Epidemiology of primary sclerosing cholangitis in general and IBD populations: a systematic review and meta-analysis

Primary Sclerosing Cholangitis (PSC) is a chronic cholestatic liver condition that is closely associated with inflammatory bowel disease (IBD). PSC is associated with substantial long-term hepatobiliary morbidity, including an increased risk of cholangiocarcinoma. Reported prevalence and incidence estimates of PSC vary widely across different populations and study designs. This meta-analysis aims to provide an updated estimate of the prevalence of PSC in the general and IBD populations, and PSC incidence in the general population.

A systematic search of MEDLINE (Ovid) and Embase from inception to October 20, 2025, for population- or cohort-based studies reporting PSC prevalence or incidence was performed. Seventy-four studies met the inclusion criteria. Random-effects meta-analyses were performed to pool prevalence and incidence estimates. Subgroup analyses, meta-regression, and sensitivity analyses were conducted to assess robustness and sources of heterogeneity.

Seventeen studies including 196,635,709 individuals showed a pooled overall PSC prevalence of 8.06 per 100,000 (95% CI 4.75-13.36) in the general population. Among 516,548 patients with IBD in thirty-four studies, pooled PSC prevalence was 15.2 per 1,000 (95% CI 10.7-21.7). PSC prevalence was higher in ulcerative colitis (20.6 per 1,000) than in Crohn's disease (10.2 per 1,000). Using Crohn's disease as the reference group, ulcerative colitis was associated with higher odds of PSC (OR 1.787, 95% CI 1.066-2.996, p = 0.028). Thirteen studies reported a pooled incidence of PSC in the general population of 0.892 per 100,000 person-years (95% CI 0.705-1.130).

This meta-analysis provides an updated synthesis of PSC epidemiology, extending prior work by incorporating detailed subgroup analyses across IBD phenotype, PSC subtype, geographic region, socioeconomic context, diagnostic modality, case-ascertainment method, and time period, which are dimensions not comprehensively addressed in previous meta-analyses. PSC remains a rare disease in the general population but is substantially more prevalent among individuals with IBD. Marked geographic and methodological heterogeneity, with notable underrepresentation of data from lower-SDI regions and the African Region, underscores the need for standardized case definitions and improved epidemiological surveillance across diverse settings. Given the markedly increased risk of cholangiocarcinoma in PSC, these findings highlight the clinical importance of recognizing PSC in higher-risk IBD populations and provide an epidemiological foundation for future studies evaluating risk stratification, early detection, and surveillance.